title: Ciliary body medulloepithelioma: analysis of 41 cases.
abstract: OBJECTIVE
  To describe the clinical features, histopathology, treatment, and outcomes of ciliary body medulloepithelioma.
  
  
  METHODS
  Retrospective study.
  
  
  METHODS
  Forty-one patients with medulloepithelioma.
  
  
  METHODS
  Cryotherapy, plaque radiotherapy, external beam radiotherapy, tumor removal by partial lamellar sclerouvectomy (PLSU), or enucleation.
  
  
  METHODS
  Metastasis and death.
  
  
  RESULTS
  Of 41 patients with ciliary body medulloepithelioma, the median age at diagnosis was 5 years. The mean tumor basal diameter was 11 mm, and the mean tumor thickness was 7 mm. Related features included secondary glaucoma (n = 18, 44%), iris neovascularization (n = 21, 51%), cataract (n = 19, 46%), lens subluxation (n = 11, 27%), lens coloboma (n = 8, 20%), retrolental neoplastic cyclitic membrane (n = 21, 51%), intratumoral cysts (n = 25, 61%), and extraocular extension (n = 4, 10%). There was systemic association with pleuropulmonary blastoma in 2 cases (5%). Primary tumor treatment included enucleation (n = 21, 60%), tumor removal by PLSU (n = 8, 23%), plaque radiotherapy (n = 3, 9%), external beam radiotherapy (n = 1, 3%), cryotherapy (n = 1, 3%), or palliative chemotherapy (n = 1, 3%). In 1 case, medulloepithelioma was diagnosed histopathologically after inadvertent evisceration for blind painful eye. Subsequent treatment for residual or recurrent tumor in cases treated conservatively/inappropriately (n = 15) was necessary in 7 cases (47%). Histopathology disclosed benign features in 6 cases (20%), malignant features in 24 cases (80%), teratoid features in 11 cases (37%), and nonteratoid features in 19 cases (63%). In the 26 enucleated eyes, other features included retrolental neoplastic cyclitic membrane (n = 18, 69%), neoplastic epiretinal membrane (n = 6, 23%), and persistent hyaloid artery (n = 6, 23%). Systemic metastasis occurred in 3 cases (8%) over a mean follow-up of 49 months, all of whom presented with extrascleral extension of tumor due to mean delay in diagnosis by 39 months.
  
  
  CONCLUSIONS
  Medulloepithelioma most commonly occurs in children. Systemic association with pleuropulmonary blastoma rarely is found. Patients with extrascleral medulloepithelioma are at risk for metastasis.
authors.affiliation: Ocular Oncology Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, Pennsylvania; The Department of Pathology, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, Pennsylvania; Ocular Oncology Service, Hyderabad, India.
authors.forename: Swathi
authors.initials: S
authors.lastname: Kaliki
authors.affiliation: Ocular Oncology Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, Pennsylvania. Electronic address: carol.shields@shieldsoncology.com.
authors.forename: Carol L
authors.initials: CL
authors.lastname: Shields
authors.affiliation: The Department of Pathology, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, Pennsylvania.
authors.forename: Ralph C
authors.initials: RC
authors.lastname: Eagle
authors.affiliation: The Department of Pathology, L. V. Prasad Eye Institute, Hyderabad, India; The Department of Pathology, University of Hyderabad, Hyderabad, India.
authors.forename: Geeta K
authors.initials: GK
authors.lastname: Vemuganti
authors.affiliation: Ocular Oncology Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, Pennsylvania.
authors.forename: Ana
authors.initials: A
authors.lastname: Almeida
authors.affiliation: Ocular Oncology Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, Pennsylvania.
authors.forename: Fairooz P
authors.initials: FP
authors.lastname: Manjandavida
authors.affiliation: The Department of Pathology, L. V. Prasad Eye Institute, Hyderabad, India.
authors.forename: Kaustubh
authors.initials: K
authors.lastname: Mulay
authors.affiliation: Ocular Oncology Service, Hyderabad, India.
authors.forename: Santosh G
authors.initials: SG
authors.lastname: Honavar
authors.affiliation: Ocular Oncology Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, Pennsylvania.
authors.forename: Jerry A
authors.initials: JA
authors.lastname: Shields
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D001918:Brachytherapy; D002648:Child; D002675:Child, Preschool; D002924:Ciliary Body / Q000473:pathology*; D017679:Cryotherapy; D015353:Eye Enucleation; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D008875:Middle Aged; D018242:Neuroectodermal Tumors, Primitive / Q000473:pathology* / Q000628:therapy; D013508:Ophthalmologic Surgical Procedures; D061766:Proton Therapy; D012189:Retrospective Studies; D016896:Treatment Outcome; D014604:Uveal Neoplasms / Q000473:pathology* / Q000628:therapy; D055815:Young Adult